Older age onset of systemic sclerosis - accelerated disease progression in all disease subsets
Open Access
- 1 November 2020
- journal article
- research article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 59 (11), 3380-3389
- https://doi.org/10.1093/rheumatology/keaa127
Abstract
Objectives. Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype. We here investigate the relationship between age at disease onset and symptoms in a large cohort of SSc patients (IcSSc, dcSSc and SSc-overlap syndromes). Methods. Clinical data of the registry of the German Network for Systemic Scleroderma including 3281 patients were evaluated and subdivided into three age groups at disease onset (60 years). Results. Among all SSc patients, 24.5% developed their first non-Raynaud phenomenon symptoms at the age 60 years), except for gastrointestinal and musculoskeletal involvement. No significant difference was found for the use of corticosteroids. However, significantly, fewer patients older than 60 years received immunosuppressive treatment. Conclusion. In this large registry, similar to 25% of patients developed SSc at an age above 60years with an increased frequency of IcSSc. In this age group, an onset of internal organ involvement was significantly accelerated across all three subsets. These findings suggest that, in the elderly cohort, more frequent follow-up examinations are required for an earlier detection of organ complications.Funding Information
- German Federal Ministry of Education and Research
- BMBF (01GM0310 NH, 01GM0631 CS)
- Edith-Busch-Foundation
This publication has 31 references indexed in Scilit:
- Late-age Onset Systemic SclerosisThe Journal of Rheumatology, 2011
- SclerodermaThe New England Journal of Medicine, 2009
- A score of risk factors associated with ischemic digital ulcers in patients affected by systemic sclerosis treated with iloprostClinical Rheumatology, 2009
- The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvementRheumatology, 2008
- Morbidity and mortality of patients diagnosed with systemic sclerosis after the age of 75: a nested case-control studyClinical Rheumatology, 2006
- Systemic sclerosis in the elderlyClinical Rheumatology, 1992
- Scleroderma in the elderly.BMJ, 1981
- Systemic sclerosis in old age.BMJ, 1979
- Progressive systemic sclerosis in the elderlyHeart, 1979